Glomerulopathies Ig A nephropathy Pathogenesis Glomerulopathies Ig A

Glomerulopathies –Ig. A nephropathy - Pathogenesis Mesangial Ig. A promotes glomerular inflammation a series

Glomerulopathies –Ig. A nephropathy - Pathogenesis What specific mechanisms influence the deposition of Ig.

Glomerulopathies –Ig. A nephropathy - Pathogenesis tightly T cell controlled processes in health most

Glomerulopathies –Ig. A nephropathy - Pathogenesis in Ig. A nephropathy synthesis of polymeric Ig.

Glomerulopathies –Ig. A nephropathy - Pathogenesis Yes it is

Glomerulopathies –Ig. A nephropathy - Pathogenesis Why might this happen? This process is very

Glomerulopathies –Ig. A nephropathy - Pathogenesis in Ig. A nephropathy - these processes is

Glomerulopathies –Ig. A nephropathy - Pathogenesis Abnormal Ig. A 1 molecule promote Ig. A

Glomerulopathies –Ig. A nephropathy - a great clinical heterogeneity

Glomerulopathies –Ig. A nephropathy is often presenting as recurrent visible hematuria coinciding with mucosal

Glomerulopathies –Ig. A nephropathy a few patients will present nephrotic proteinuria

Glomerulopathies –Ig. A nephropathy other patients will be picked up because they have an

Glomerulopathies –Ig. A nephropathy others will already have advanced kidney disease when they’re first

Glomerulopathies –Ig. A nephropathy “Is this one disease? Is this the same disease we’re

Glomerulopathies –Ig. A nephropathy in a global view of this disease it’s extremely different

Glomerulopathies –Ig. A nephropathy also a very variable prognosis

Glomerulopathies –Ig. A nephropathy - prognosis proteinuria, hypertension and GFR at the time of

Glomerulopathies –Ig. A nephropathy – prognosis glomerulosclerosis, interstitial fibrosis, tubular atrophy predict outcome that’s

Glomerulopathies –Ig. A nephropathy – prognosis proteinuria you have not just at presentation but

Glomerulopathies –Ig. A nephropathy – prognosis how much proteinuria you have during follow up

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura What is Ig. A nephropathy? characterised

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura a more accurate definition of H.

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura Ig. A deposits in the mesangium

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura a typical picture of Ig. A

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura A more aggressive type of Ig.

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura C 3 is also found in

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura A more aggressive case - massive

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura H. S. Purpura is generally a

Glomerulopathies –Ig. A nephropathy management recommendations

Glomerulopathies –Ig. A nephropathy management recommendations There is no specific treatments for this condition

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Glomerulopathies –Ig. A nephropathy - Pathogenesis

Glomerulopathies –Ig. A nephropathy - Pathogenesis Mesangial Ig. A promotes glomerular inflammation a series of events ……. can cause end stage renal disease

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis What specific mechanisms influence the deposition of Ig. A and its initiation of inflammation?

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis tightly T cell controlled processes in health most polymeric Ig. A is produced in the mucosal immune system is involved in mucosal defense the bone marrow produces monomeric Ig. A, which reaches the circulation

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis in Ig. A nephropathy synthesis of polymeric Ig. A is increased in the bone marrow and decreased in the mucosa

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis Yes it is

Glomerulopathies –Ig. A nephropathy - Pathogenesis Why might this happen? This process is very tightly T cell regulated In response to a mucosal antigen, T cells reach the circulation, but will return to the mucosa to synthesize polymeric Ig. A if they express alpha 4 beta 7, the mucosal homing receptor If on the other hand, a systemic antigen is confronted, T cells will home to the marrow if they express the alpha 4 beta 1 systemic homing receptor

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis in Ig. A nephropathy - these processes is abnormal there is an exaggeration of systemic homing receptor expression, which would drive T cells not back to the mucosa but towards the marrow the abnormal site of Ig. A 1 production, which is one of the features of Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis in Ig. A nephropathy - these processes is abnormal but not only there is an exaggeration of systemic homing receptor expression, which would drive T cells not back to the mucosa but towards the marrow the abnormal site of Ig. A 1 production, which is one of the features of Ig. A nephropathy structural abnormalities of the Ig. A molecule itself

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - Pathogenesis Abnormal Ig. A 1 molecule promote Ig. A 1 self-aggregation production of macromolecular Ig. A 1 act as an antigen production of Ig. A 1 -Ig. G complexes, which could be deposited influence interaction directly with matrix components and particularly with mesangial cells

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - a great clinical heterogeneity

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy is often presenting as recurrent visible hematuria coinciding with mucosal infection which is almost never seen over the age of 40

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy a few patients will present nephrotic proteinuria

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy other patients will be picked up because they have an asymptomatic urine abnormality

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy others will already have advanced kidney disease when they’re first picked up

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy “Is this one disease? Is this the same disease we’re picking up at different stages? ” Prof J. Feehally Dept of Nephrology Leicester General Hospital Leicester, United Kingdom

Glomerulopathies –Ig. A nephropathy in a global view of this disease it’s extremely different in its prevalence

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy also a very variable prognosis

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy - prognosis proteinuria, hypertension and GFR at the time of diagnosis predict outcome that’s true for any glomerular disease

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy – prognosis glomerulosclerosis, interstitial fibrosis, tubular atrophy predict outcome that’s true for any glomerular disease

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy – prognosis proteinuria you have not just at presentation but during prolonged follow up is a very powerful predictor of outcome !!

Glomerulopathies –Ig. A nephropathy – prognosis how much proteinuria you have during follow up combined with mean arterial pressure during follow up are the most powerful predictor of outcome

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura What is Ig. A nephropathy? characterised by predominant mesangial Ig. A deposits by immunofluorescence on the renal biopsy there is no clinical nor serological features, which are specific for Ig. A nephropathies to make the diagnosis, you really need a renal biopsy with appropriate immunofluorescence What is Henoch Schonlein Purpura? a true clinical syndrome characterised by palpable purpura, arthritis, and abdominal pain, and of course, a glomerulonephritis characterised by predominant Ig. A deposits

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura a more accurate definition of H. S. Purpura a small vessel vasculitis with Ig. A dominant deposits involving skin, gut, and glomeruli associated with arthritis

Glomerulopathies –Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura Ig. A deposits in the mesangium –for sure it is a primary Ig. A nephropathy or a H. S. Purpura rule out a systemic disease associated with Ig. A deposits

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura a typical picture of Ig. A deposits characterized by granular mesangial deposits of Ig. A

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura A more aggressive type of Ig. A nephropathy üassociated with much more massive immunoglobulin deposits of Ig. A üthere is thorough extension of Ig. A deposits along the capillary walls üis usually associated with proliferative lesion and endocapillary proliferation

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura C 3 is also found in the mesangium generally with a more or less identical pattern than for Ig. A – generally the intensity is a little bit weaker

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura A more aggressive case - massive deposits of C 3 in mesangial area, and also extension along the capillary walls

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura H. S. Purpura is generally a more aggressive type of glomerulonephritis than Ig. A nephropathy

Glomerulopathies –Ig. A nephropathy and Henoch Schonlein Purpura

Glomerulopathies –Ig. A nephropathy management recommendations

Glomerulopathies –Ig. A nephropathy management recommendations There is no specific treatments for this condition

Glomerulopathies –Ig. A nephropathy management recommendations

Glomerulopathies –Ig. A nephropathy

Glomerulopathies